A metabolic disease associated with gluconeogenesis is glycogen storage disease type I, also known as von Gierke disease, which belongs to the glycogenoses. Type Ia is due to a defect of glucose-6-phosphatase, whereas type Ib concerns glucose-6-phosphate translocase. Both cases result in a defective conversion of glucose-6-phosphate into glucose.
Outline:
00:00 - Introduction
02:19 - Reactions
08:20 - Clinical relevance
09:08 - Summary
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